Topic outline

  • Grand Rounds

    Tolosa Hunt Syndrome is an idiopathic inflammatory process of the cavernous sinus of unknown etiology. There are no known risk factors, but an inciting event, such as a viral illness, vaccination, trauma, or tumors, are known to be preceding events that result in nonspecific inflammation of the cavernous sinus. The International Classification of Headache Disorders has published clinical, histopathological, and radiographic criteria to aid in the diagnosis of the disease. Patients typically present with ipsilateral orbital pain or headaches that precede cranial nerve palsies by less than 2 weeks. Cranial neuropathies typically involve CNs III, IV, V, and VI. Less commonly, the optic nerve, sympathetic or parasympathetic nervous systems can be involved. Neuroimaging has been pivotal in the diagnosis of the disease and typically shows abnormal tissue that is isointense on T1 images and isointense to slightly hypointense on T2 weighted images. A patient’s response to steroid treatment is also critical in making the diagnosis: patients typically have resolution of their orbital pain within 24-72 hours of steroid initiation. Cranial nerve palsies and neuro-imaging findings typically resolve a few weeks later. Finally, all patients with clinical and radiographic signs of Tolosa Hunt Syndrome must complete bloodwork and CSF analysis to exclude other inflammatory, infectious, or neoplastic etiologies. These patients must be followed closely to ensure resolution of their disease process. Here, we present a case of Tolosa Hunt Syndrome in a patient with ptosis, upgaze, and adduction deficits, whose disease process resolved on oral prednisone, but recurred several months later with variable clinical findings.

    Presentation Date: 03/16/2023
    Issue Date: 03/31/2023